Renal manifestations of tuberous sclerosis complex.
angiomyolipoma; autosomal polycystic kidney disease; renal cystic disease; tuberous sclerosis; Von Hippel–Lindau disease
Tuberous sclerosis complex (TSC) is a genetic condition caused by a mutation in either the TSC1 or TSC2 gene. Disruption of either of these genes leads to impaired production of hamartin or tuberin proteins, leading to the manifestation of skin lesions, tumors, and seizures. TSC can manifest in multiple organ systems with the cutaneous and renal systems being the most commonly affected. These manifestations can secondarily lead to the development of hypertension, chronic kidney disease, and neurocognitive declines. The renal pathologies most commonly seen in TSC are angiomyolipoma, renal cysts, and less commonly, oncocytomas. In this review, we highlight the current understanding on the renal manifestations of TSC along with current diagnosis and treatment guidelines. (Copyright: Nair N et al.)
Nair N;Chakraborty R;Mahajan Z;Sharma A;Sethi SK;Raina R
Journal Of Kidney Cancer And VHL
2020
2020-08-27
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.15586/jkcvhl.2020.131" target="_blank" rel="noreferrer noopener">10.15586/jkcvhl.2020.131</a>
Challenges of long-term vascular access in pediatric hemodialysis: Recommendations for practitioners.
Pediatrics; hemodialysis; vascular access; central venous catheter; arteriovenous grafts
Kidney transplantation is the preferred treatment of end-stage renal disease in children. However, time to transplant varies, making a well-functioning long-term vascular access essential for performing hemodialysis efficiently and without disruption until a kidney becomes available. However, establishing long-term vascular access in pediatric patients can present distinct challenges due to this population's unique characteristics, such as smaller body size and lower-diameter blood vessels. There are three main pediatric long-term vascular access options, which include central venous catheters (CVC), arteriovenous fistula (AVF), and arteriovenous graft (AVG). CVC are currently the most widely used modality, although various studies and guidelines recommend AVF or AVG as the preferred option. Although AVF should be used whenever possible, it is crucial that clinicians consider factors such as patient size, physical exam findings, comorbidities, predicted duration of treatment to decide on the most optimal long-term vascular access modality. This article reviews the three long-term vascular access methods in children and the benefits and complications of each.
Raina R;Joshi H;Chakraborty R;Sethi SK
Hemodialysis International.
2020
2020-10-18
journalArticle
<a href="http://doi.org/10.1111/hdi.12868" target="_blank" rel="noreferrer noopener">10.1111/hdi.12868</a>
Hypertensive crisis in pediatric patients: An overview.
management; acute severe hypertension; hypertensive crisis; hypertensive emergency; hypertensive urgency
Hypertensive crisis can be a source of morbidity and mortality in the pediatric population. While the epidemiology has been difficult to pinpoint, it is well-known that secondary causes of pediatric hypertension contribute to a greater incidence of hypertensive crisis in pediatrics. Hypertensive crisis may manifest with non-specific symptoms as well as distinct and acute symptoms in the presence of end-organ damage. Hypertensive emergency, the form of hypertensive crisis with end-organ damage, may present with more severe symptoms and lead to permanent organ damage. Thus, it is crucial to evaluate any pediatric patient suspected of hypertensive emergency with a thorough workup while acutely treating the elevated blood pressure in a gradual manner. Management of hypertensive crisis is chosen based on the presence of end-organ damage and can range from fast-acting intravenous medication to oral medication for less severe cases. Treatment of such demands a careful balance between decreasing blood pressure in a gradual manner while preventing damage end-organ damage. In special situations, protocols have been established for treatment of hypertensive crisis, such as in the presence of endocrinologic neoplasms, monogenic causes of hypertension, renal diseases, and cardiac disease. With the advent of telehealth, clinicians are further able to extend their reach of care to emergency settings and aid emergency medical service (EMS) providers in real time. In addition, further updates on the evolving topic of hypertension in the pediatric population and novel drug development continues to improve outcomes and efficiency in diagnosis and management of hypertension and consequent hypertensive crisis.
Raina R;Mahajan Z;Sharma A;Chakraborty R;Mahajan S;Sethi SK;Kapur G;Kaelber D
Frontiers in Pediatrics
2020
1905-07
journalArticle
<a href="http://doi.org/10.3389/fped.2020.588911" target="_blank" rel="noreferrer noopener">10.3389/fped.2020.588911</a>
Development of acute kidney injury following pediatric cardiac surgery.
Pediatrics; Acute kidney injury; Nephrology; Thoracic surgery
Acute kidney injury (AKI) in the pediatric population is a relatively common phenomenon. Specifically, AKI has been found in increasing numbers within the pediatric population following cardiac surgery, with up to 43% of pediatric patients developing AKI post-cardiac surgery. However, recent advances have allowed for the identification of risk factors. These can be divided into preoperative, intraoperative, and postoperative factors. Although the majority of pediatric patients developing AKI after cardiac surgery completely recover, this condition is associated with worse outcomes. These include fluid overload and increased mortality and result in longer hospital and intensive care unit stays. Detecting the presence of AKI has advanced; use of relatively novel biomarkers, including neutrophil gelatinase associated lipocalin, has shown promise in detecting more subtle changes in kidney function when compared to conventional methods. While a single, superior treatment has not been elucidated yet, novel functions of medications, including fenoldopam, theophylline and aminophylline, have been shown to have better outcomes for these patients. With the recent advances in identification of risk factors, outcomes, diagnosis, and management, the medical community can further explain the complexities of AKI in the pediatric population post-cardiac surgery.
Sharma A; Chakraborty R; Sharma K; Sethi SK; Raina R
Kidney Research and Clinical Practice
2020
2020-08-10
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
journalArticle
<a href="http://doi.org/10.23876/j.krcp.20.053" target="_blank" rel="noreferrer noopener">10.23876/j.krcp.20.053</a>